So Many Changes in a Few Short Months

Our family has been very busy keeping up with lots of moving parts in this journey we are on! Since my post in July Rayleigh has started school, is completely off Banzel, now gets real food through her feeding tube instead of formula, is on a slightly increased dose of Charlotte's Web, had family photos done, went to Disney on Ice, has in-home nursing, went on her first field trip, and is getting a wish granted by Make a Wish of Oklahoma! 

Whoa.

Real quick, here are some of my favorites from this year's family photo session!

Family photos for us are bittersweet. We love love love capturing our beautiful girl in pictures to make memories that will last forever. On the other hand, it is one of those things that makes us painfully aware of her disabilities. Obviously, no one is more aware of her disabilities than Michael and I, but we are so involved every single day that it has just become life. A beautiful, meaningful life that we love belonging to. But several things in life make us think, "Dang, that was hard". For family photos, it was because we couldn't just sit her on the ground and tell her to smile, or pick any pose we like. Every single pose had to be thought through: "How will we hold Rayleigh?", "Can't do that, she doesn't have enough head control", "She's too big for that kind of pose but not able to do the older-kid kind of poses"... 
Luckily, we had a wonderful and very patient photographer who would allow us to get in poses we typically do with Rayleigh or ones I found online that I thought we could do. Some worked out great, others... not so much. Nevertheless, I am so pleased with this year's batch of family photos and am so grateful to have these memories! 


Now to the update: Poor little Rayleigh's body is still adjusting to the medicine wean of Banzel. It has been about 1 month since her last dose. We weaned very slowly and the wean went really well. We saw very little withdrawal, and when seizures would increase from withdrawal she would stabilize and get to her baseline after only a couple of days. She is still very tired after seizures and even when she only has a few a day, the post-seizure-naps can wreck up a day. We tried to take her to the science museum on Monday but as soon as we checked in she had her first big seizure of the day so we turned around and came home so she could get a good nap. This is not abnormal for our life.

After the final dose of Banzel we saw withdrawal symptoms come and go for several weeks. At this point, she has had 4 good days in a row so we are hopeful that she is fully stabilizing (knock on wood!). She is having less than 5 seizures on a good day, but still up to 10 of her bigger 3 minute complex partial seizures on a bad day. Two weeks ago we increased her Charlotte's Web Hemp Oil by a tiny amount, Rayleigh starts to show changes from the hemp oil at around 3 weeks so I'm excited to see what comes about - because, again, she has had 4 good days in row ;) 

School is going well! Buggie currently goes half days as we don't think she could tolerate a full day of activities right now. She loves the music, art, circle time, and of course being around people! 

First Day of School - Yes, she was asleep when we left home!

But she woke up when class was about to begin!

Her first field trip was last week and I think she really enjoyed it! It was a windy day at a Pumpkin Patch farm. Rayleigh got to pet different animals, pick out pumpkins and go on a hayride with her school friends!

I'll do a separate post on Disney on Ice and Rayleigh's Make a Wish. They are too much fun to squeeze into an update-post! I'll just leave you with this sneak peek picture from the day we met Rayleigh's sponsors! 

The Perfect Summer Day

Yesterday was the Fourth of July; our flag was proudly flying in the wind in front of our house, the sun was shining, and the heat was on! 

Rayleigh's dad had to work a little in the morning, so Bug and I lounged in bed and watched cartoons for a little bit and then went to the backyard to read and enjoy the beginning of what would be a wonderful day! 

Mommy & Bug Backyard Selfie :) 

Next, because of a seizure, Rayleigh took a nice nap in the cool air conditioning. When she woke up we headed to the mall for an afternoon movie! This was sort of spur of the moment, but our girl was in a great mood and I've been wanting to try the movie theater again. We've only taken Bug to the theater once and it was a disaster! We took her to a very early showtime of Despicable Me 2 for a sensory show - and being in withdrawal from weaning a medicine she was cranky and miserable and just wanted to be home, so we left about 30 minutes into the movie. But that was a couple of years ago and she has been having some really good days so we thought LET'S TRY! 

And we are SO GLAD we did!! She loved it, y'all! We went to see Inside Out! The theater we chose has the wide recliner seats and we were able to get 2 spots and wheelchair spot. Rayleigh watched the first bit in her wheelchair and then snuggled up with her blanket on my lap and we raised the armrest so she could be close to her dada, too! We did her afternoon tube feeding right there, easy peasy. Goodness, I'm still so proud of her, and so happy she was able to enjoy the movie!!! 

Movie Theater Selfie!

We went over to her grandparents' house after the movie for dinner and some swimming! 

We sent Daddy for sunglasses after her snapped a picture! hehe!

Then, of course, fireworks!! We went to a park in a nearby city where loads of people go to pop off their own fireworks! Rayleigh managed to stay awake long enough to watch her dad and Gpa shoot off some pretty ones, but then she fell asleep snuggled on my lap. Her awesome Lovie sat in the air conditioned van with her to finish watching the boys. 
Sorry I didn't take more pictures, I notice at the end of a fun day that I don't take very many pictures of those moments we had - I was so busy living life and enjoying the moment that I forgot to whip out my phone and snap a quick picture to remind me of the fun we had! I'll work on getting better, haha!

I am just soo thankful that Rayleigh got to enjoy the holiday! Today, she is making up for all of that spent energy with a big long nap (hence the time for this update!). 

Home Sweet Homa

Hmmm... Where to start? I guess I'll just dive right in. After months and months of Michael trying to transfer his job to Colorado we thought, "Well, maybe there's a reason this isn't falling in to place despite our efforts." 

That's the small thought that led to a grand discussion and ultimately, the decision to stay in Oklahoma. At least for the foreseeable future. 

Our decision was not made lightly but once it was spoken aloud, there was a wave of calm that went through me. It felt like I had finally released a breath that I had been holding in for a very long time. 

Rayleigh is the most important thing in my life. Do I think it would be beneficial for her to have different options in CBD oils and access to THC oil to use as a supplement to the CBD oils? Sure. Do I think the answer is to uproot and move to Colorado for their supply? Not right now. We have seen some great stuff from high CBD oil use. In fact, Rayleigh is currently taking Charlotte's Web Hemp Oil. She is still having 6-10 complex partial seizures a day, they are still wearing out her little body, BUT she is having random days of only 2 or 3 of these seizures. And, no joking, her eyes are so much more alert. The girl is AWAKE when she is AWAKE! We just recently increased the dose (you have to titrate your way up to an optimal dose slowly) and we have seen a little bit of movement coming back, mostly wiggling her arms or moving her head! 

So, I'm not sure if high CBD oil is going to be our answer for seizure control or not.
But I'm going to keep on trying until I have reason to stop. 

To everyone that donated toward our Colorado move: THANK YOU! You have such loving hearts. That money was set aside and has been/is being used to buy the Charlotte's Web Hemp Oil. Each bottle is $250 plus shipping, so your help is soo appreciated! 

Oklahoma recently passed a bill that allows only high CBD oils to patients under 18 with intractable epilepsy. Rayleigh fits the criteria (duh.) and has her neurologist's blessing. I've got some issues with the new law, the biggest being: How the heck are the patients to get the oil when it's still federally illegal for the providers to ship any form of medical cannabis outside of their state? 

{Charlotte's Web was classified as Hemp Oil this past year and is currently shipping to all 50 states under that pretense. All other high CBD medical cannabis oils that I know of are NOT shipping nor classified as Hemp.}

After Oklahoma passed the bill OU Medical Center announced that they will begin the Epidiolex trial. This is for a big pharma version of medical cannabis, it is very high in CBD and extremely low in THC. I have heard CDKL5 success stories from the clinical trials of Epidiolex in other states so I am going to contact the coordinator and inquire about getting Rayleigh into the study. I will not put her in the study if it is controlled with a placebo. Why would I take her off a for sure high CBD hemp oil to put her in a study with the possibility that she gets the placebo?

In closure, we have a plan with her neurologist to wean her Banzel (one of her seizure medicines). Once Banzel is out of her system we will start either Fycompa or Sabril. She's been on Sabril before with good seizure control but some side effects, she's never been on Fycompa and while it's only been available for about a year I'm hearing good things. Decisions. Hopefully the medicine that we decide on and the CWHO will give Rayleigh enough seizure control that we can get back to weaning Onfi (the monster seizure medicine). 

Thank you for your patience and love while we were in limbo about the move. 
Oklahoma, you have a strong hold on this family!

And now, some pictures from the past year!

Return of the Tonic Seizures

I haven't  posted in a while because everything around here has been busy and we have been in quite a routine and there wasn't really anything major to report but unfortunately, that did not last long.

Just over a week ago Rayleigh started having tonic seizures again. This is where her body tenses and her breathing slows drastically. Her face looks worried while it happens which is the most heartbreaking part. Her seizures before this were more like head drops, pauses and other 1-3 second seizures. These tonics last about 30 seconds total give or take. We use her VNS magnet as soon as we notice the seizure and that usually shortens the seizure or stops it all together but sometimes it doesn't seem to help with the seizure at all. Bug is having about 5 of these tonics a day.

When they first started it was very stormy here in Oklahoma and we attributed the increased seizure activity to the barometric weather pressures because it always seems to have some kind of negative effect on her seizures. But we soon realized, in between storms, that this was much too worse to be caused by weather changes. 

We spoke with her neuro and he said we may need to make some medication or VNS adjustments but first he wanted us to try using her Clonazepam PRN, 1 pill twice a day for 3-5 days. After 2 full days on the Clonazepam we noticed a decrease of seizure activity BUT her mood was inconsolable. She was literally irritable and uncomfortable all day long, so we didn't do any Clonazepam on the 3rd day and she started getting calmer and happier through the day and the next day. But today, she is unhappy again.

We've doing rounds of Tylenol/Advil and providing lots of stimulation by rocking, bouncing, swinging, and snuggling but everything is only temporary relief. Her neuro isn't back in the office until Wednesday and we see him then but I, being mommy, want to fix her now. Moms know that there is no worse feeling than knowing your child is hurting and you can't do anything about it.

I don't normally reach out for prayer request but we could use some now. Please pray for Rayleigh's comfort to return and for her pain to vanish - and pray that Michael and I can find a way to keep our girl happy until we can see the doctor about this. Thank you so very much :)

Uncertain Why

Michael and I are at a loss. Over the last few weeks we have noticed Bug's seizure activity changing. At first it was just like her partial onset seizures at night were going away and were being replaced with 1-3 atonic head drop seizures. Her upper body would just kind of collapse for a split second and then she would pop right back up. This is different from the myclonic spasms she was having a year ago where her arms would spread out wide and she would quickly bend down so we (her neuro, Michael and I) do not think it is epileptic spasms again which is good because those are so difficult to control.

When the atonic head drops started we weren't too super concerned because were confidant that raising the strength of her VNS pulses and changing the duty cycle, discussed in the previous post, would zap away those last few drop seizures. Well, changing the cycle has seemed to completely control the partial onset seizures (fingers crossed!) but now the atonic head drops are increasing and coming in clusters.

I've been keeping all of the notes in Bug's seizure journal as usual and I just don't know what to make of it. We started a medicine a few weeks ago to control excessive drool but the neuro doesn't think that would attribute to the increase of drops.

I had a sickening feeling that he is going to want an EEG soon and Rayleigh just does NOT do well with the sleep deprivation involved with an EEG and doesn't always nap once we get there for the appointment. But if it is necessary then we will do it!

We see the neuro this week and I am really hoping that he believes we can tweak the settings on the VNS and get these drops under better control. After a cluster of 3-5 drops within a 3 minute period Bug gets fussy. She sounds uncomfortable, not scared or hurt, but as a mom you still want to "fix" whatever is ailing your child.

I really hope we can fix this issue before it gets any worse. She is my angel, my world.

--------------------------
Current meds:
Onfi 10mg, 1 pill, 3 times a day
Vimpat 50mg, 1/2 pill morning, 1 pill night
Banzel, 2ml twice a day
Kuspova (drool med), 2.5ml twice a day

VNS set at 1.25 out of 2 strength, on for 30 seconds every 3 minutes

Our Experience with Banzel

Maybe you're thinking that the title of this should be "Bug's Experience..." but let me tell you, any time we make a change for Bug, we all go through it! You'll see what I mean in this little story.

Banzel is a medication designed to control seizures in combination with other medicines. Rayleigh Bug started taking Banzel on November 13th. We began with 1ml twice a day for one week, then 2ml twice a day for one week and then reached her goal dose of 3ml twice a day. She is also taking Clobozam/Onfi and Vimpat for seizure control.

Not long after she had been on the full dose did we start noticing mood changes. I mean, she hadn't been her happy, bubbly self since Lamictal months ago, which she stopped taking at the beginning of November - but this change was awful. I don't mean to be the gray rain cloud over all this but it was bad! She literally woke up cranky making whiny noises, irritable, kicking, uncomfortable, flailing, and such.

So we figured, "Ok, this is probably just her system adjusting to the new dose of Banzel. It will wear off as her body gets used to it. I hope."

2 weeks passed and we couldn't stand it any longer. Our little girl was not happy. BUT she had the best seizure control she had obtained in months! Before Banzel she was having 2-4 tonic seizures a day. A tonic seizure being where she would tense and curl her limbs in with good breathing or sometimes labored breathing and her eyes would look off, no shaking or jerks. Each tonic seizure lasted about 60-70 seconds.

With the full dose of Banzel in her system, Bug was only having a few tonic seizures a week and had started having absent seizures. An absent seizure for Rayleigh involves her looking off, usually to her left, for 1-2 seconds and then coming right back to. She was having about 2-3 of these new seizures a day after Banzel.

I contacted Bug's neuro and gave him our list of pros and cons and he explained that everything I was describing were side effects of Banzel. He prescribed us to lower the dose of Banzel from 3ml to 2ml twice a day and wait a few weeks for changes to be seen.

So we waited.

And we waited.

And we kept waiting.

I am very very very happy to report that her mood has MUCH IMPROVED these last couple of weeks! She's laughing, giggling, making eye contact, waking up and snuggling instead of kicking and she is just happy!

We're still not sure if it was the Banzel finally adjusting in her system with the smaller dose or if the VNS being activated had something to do with it but we are so grateful that she is feeling better!

So anyway, my advice to parents considering Banzel: The first few months are tough so make a glass of wine and let it pass and do whatever makes them happy (for Rayleigh during those first months we would drive around, the motion would momentarily calm her) and if you see seizure control then try and stick with Banzel and the irritability should wear off, eventually ;)

Rescheduled With No Date

I thought I would be posting more about Rayleigh's VNS by now but that will have to wait. Unfortunately, her surgery has been pushed back again. It was originally scheduled for November 29th but our secondary insurance had the payment as pending so the hospital had to wait because they need it paid in full before they can operate. So we were given a new date of December 13th. Then, the neurosurgeon's office called this week to tell us that our secondary insurance is now saying that the VNS is not FDA approved for children under 12 years old and they are wanting a lot of "proof" that this is a good idea for Rayleigh. They want copies of her MRIs, EEGs and letters from her neurologist, epileptologist and neurosurgeon. I have no doubt that these letters will get written and sent promptly but I highly doubt they will be read soon.

This time, they have rescheduled us without a date. The office will call us when they hear from our secondary insurance and we will set a date then. The nurse said it will likely be in the New Year.

I don't know why this upsets me as much as it does, but I'm really sad about the whole deal. Maybe its because I'm the kind of person that goes by the calendar, maybe it's because I've always been a little sad when something I've wanted gets puts off but I really think it's because I want this for Bug so bad.

I feel like the quicker she gets the VNS put in, the faster we can

activate it and the faster we can see the results.

 

Like every day matters.

 

 

Really quickly I will let you know that Rayleigh Bug has been on the full dose of the new medicine and has been Lamictal-free for 2 weeks now. She tolerated the transition fairly well (compared to previous medicine/dose changes). She is finally starting to take naps in the middle of the day again - something that stopped when she was taking Lamictal. Bug's seizure frequency and severity has gone down in the last couple of weeks and we aren't seeing any side effects. Well, maybe crankiness but she is also cutting her last molar so it could be attributed to that...

 

 

Rayleigh's been a happier girl these days, even flashing smiles again!! And she seems stronger. I think it's a combination of hard work and putting weight back on again!

 

Photo taken in October 2012

 

She is one tough cookie!